Figure 2.

The chromatin remodeler ATRX recognizes histone-modification states and modulates chromatin dynamics in vivo. The ATRX protein contains a N-terminal ADD and C-terminal ATPase domain, which function in the regulation of its binding and enzymatic activities, respectively. The ATRX ADD recognizes multiple histone marks, including H3K4me0 and H3K9me3. Daxx, an H3.3 specificity factor, and ATRX complex together as a chaperone system specifically deposit the replication-independent histone variant H3.3 at telomeric and pericentromeric heterochromatic regions of the genome. Note: mutations in ATR-X patients map to both the ATRX-DNMT3-DNMT3L (ADD) and catalytic ATPase/helicase domains.